Curr Neurol Neurosci Rep (2017) 17:48

DOI 10.1007/s11910-017-0758-6

EPILEPSY (CW BAZIL, SECTION EDITOR)

The New Classification of Seizures by the International League

# Springer Science+Business Media New York 2017

Abstract Introduction
Purpose of Review This review presents the newly developed
International League Against Epilepsy (ILAE) 2017 classifi- Classification of seizure types is useful for several purposes.
cation of seizure types. Classification allows grouping for applying or testing thera-
Recent Findings The fundamental distinction is between sei- pies for different types of seizures. It provides a foundation for
zures that begin focally in one hemisphere of the brain, gen- basic and clinical research and epidemiological studies.
eralized onset seizures that apparently originate in both hemi- Classification provides a common language for clinicians to
spheres, and seizures of unknown onset. Focal seizures op- use to collaborate on patient care. A classification system pro-
tionally can be subclassified according to whether awareness vides words to people with epilepsy and their families to name
(a surrogate marker for consciousness) is intact or impaired. their seizure types. The classification used by most
The next level of classification for focal seizures is motor epileptologists was developed in 1981 by a committee of the
(with subgroups automatisms, atonic, clonic, epileptic spasms, International League Against Epilepsy (ILAE) [1], based up-
hyperkinetic, myoclonic, tonic), non-motor (with subgroups on review and analysis of hundreds of videotaped seizures.
autonomic, behavior arrest, cognitive, emotional, sensory), This was, in turn, a revision of an earlier classification pro-
and focal to bilateral tonic-clonic. Generalized seizures are posed by Gastaut [2].
categorized as motor (tonic-clonic, clonic, tonic, myoclonic,
myoclonic-tonic-clonic, myoclonic-atonic, atonic, epileptic Motivation for a New Classification
spasms) and non-motor/absence (typical, atypical, myoclonic,
eyelid myoclonia). The 1981 classification, which promulgated the terms simple
Summary The classification allows new types of focal sei- partial seizure, complex partial seizure, generalized tonic-
zures and a few new generalized seizures, and clarifies terms clonic seizure, absence seizure, secondarily generalized
used to name seizures. tonic-clonic seizure, and others, has been useful, but it has
limitations. The classification has no place for several focal
motor seizure types such as tonic, clonic, atonic, or myoclonic
Keywords Epilepsy . Seizure . Classification . Focal seizure . other than in a general partial motor category. Seizures are
Generalized seizure . Taxonomy unclassified if the beginning is missed, even though it may be
obvious that the seizure is tonic-clonic. The 1981 classifica-
tion does not specifically name certain important seizure
This article is part of the Topical Collection on Epilepsy
types. The words used to describe seizures, for instance com-
plex partial or psychic, may not be well understood by the
* Robert S. Fisher
[email protected]
public or non-specialist physicians. The role of consciousness
in defining a seizure type is complicated and difficult to apply
1
Department of Neurology and Neurological Sciences, Stanford
in practice. For these reasons, the ILAE commissioned a revi-
University School of Medicine, 213 Quarry Road, Palo sion of the seizure classification [3, 4] and epilepsy classi-
Alto, CA 94304-5979, USA fication [5].
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A scientific classification would be based upon the under- hemisphere and then propagate to both hemispheres. A focal
lying pathobiology of seizures and would explain why there to bilateral tonic-clonic seizure is more of a propagation pat-
are different types of seizures. Unfortunately, our current level tern than an individual seizure type, but it is such an important
of understanding does not allow construction of such a true and common type of seizure that a distinct name is justified.
classification system. Therefore, any seizure type classifica- Generalized onset seizures are categorized as motor and
tion will be operational (practical) and observational. non-motor (absence) seizures. The generalized onset tonic-
Different needs might mandate different classification sys- clonic seizure, colloquially referred to as grand mal, is well
tems. Neonatologists might develop a classification quite dif- known to the general clinician, and therefore has a place in the
ferent from one formulated by neurosurgeons, neuropharma- basic classification.
cologists, or laboratory researchers. The ILAE 2017 classifi-
cation system is directed at practicing clinicians, but en-
deavors to be of use to a wider population. The Expanded Seizure Classification

The Basic Seizure Classification The expanded seizure classification (Fig. 1b) is intended for
use by clinicians with expertise in diagnosis and treatment of
The basic seizure classification (Fig. 1a) is intended for use by epilepsy. The framework is the same as that of the basic clas-
practitioners not specializing on epilepsy. Seizures are divided sification, but specific subheadings are expanded. Motor onset
into those that begin with focal onsetlimited to one hemi- seizures optionally can be subdivided according to whether
sphere of the brainor generalized onsetwith apparent the first prominent manifestation is automatisms, atonic activ-
clinical or EEG onset in both hemispheres of the brain. The ity, clonic activity, epileptic spasms (previously called infan-
term focal replaces the previously used term partial. The tile spasms), hyperkinetic motions, myoclonic jerks, or tonic
category of unknown onset seizures is a placeholder when the stiffening.
nature of seizure onset is known with less than 80% confi-
dence by the clinician (the 80% level of confidence was arbi- Focal Onset Seizures
trarily chosen to match the usual acceptable false-negative
beta error in an experiment). An unknown onset seizure later Automatisms are robotic repetitive semi-purposeful move-
may be reclassified as focal or generalized as new information ments during a seizure, such as lip-smacking, wandering,
becomes available. hand patting and rubbing, repetitive phrases, undressing, or
Focal onset seizures are subdivided into those with retained other automatic activities. A focal atonic seizure manifests as
and impaired awareness. Focal aware seizures correspond to focal loss of tone in a limb, whereas, a focal tonic seizure
the 1981 classification simple partial seizures and focal im- entail stiffening of the limb or the neck. Clonic implies
paired awareness seizures to the previous designation com- sustained rhythmical jerking and myoclonic brief irregular
plex partial seizures. Awareness is employed as a surrogate jerking. A hyperkinetic seizure is synonymous with the term
marker for consciousness. The level of consciousness during a hypermotor seizure and may entail thrashing and pedaling.
seizure has great practical implication for predicting the im- Epileptic spasms present with flexion at the waist and flexion
pact of the seizure on behavior. Other potential surrogate or extension of the arms, usually in clusters in young children.
markers might have included level of responsiveness or mem- Careful observation of the clinical and EEG pattern may be
ory, but both of these are difficult to retrospectively assess. As required to distinguish focal onset from generalized onset ep-
a practical matter, most seizures with impaired memory also ileptic spasms. Epileptic spasms may be called infantile
demonstrate impaired awareness, except for rare pure spasms when occurring during the infantile stage of life.
amnestic seizures. Classification by level of awareness is op- Several of the focal motor categories also appear under the
tional and may be omitted if the level of awareness is either heading of generalized onset motor seizures; however, there is
unknown or does not apply, for example, with a focal myo- no implication that the pathophysiology or prognosis of sei-
clonic seizure. zures with corresponding names in each category is the same.
The next classification level of focal onset seizures is motor Focal non-motor onset seizures include autonomic sei-
versus non-motor. Subtypes of these categories are specified zures, behavioral arrest seizures, cognitive seizures, emotional
in the expanded classification system described below. A sei- seizures, and sensory seizures. A focal autonomic seizure is a
zure that starts focally and then spreads to bilateral tonic- seizure whose primary effect is on autonomic nervous system
clonic movement is called a focal to bilateral tonic-clonic sei- functions, such as heart rate, blood pressure, sweating, skin
zure, corresponding to the old phrase secondarily generalized color, piloerection, gastrointestinal sensations. A focal behav-
tonic-clonic seizure. The 2017 classification reserves the ior arrest seizure produces cessation of movement, sometimes
word generalized for seizures that are generalized from on- called a freeze or a pause. Because brief behavior arrest is
set and the word bilateral for seizures that start one common and hard to recognize as being abnormal, a seizure
Curr Neurol Neurosci Rep (2017) 17:48 Page 3 of 6 48

a ILAE 2017 Classification of Seizure Types Basic Version 1

Focal Onset Generalized Onset Unknown Onset

Impaired Motor Motor

Unclassified 2
focal to bilateral tonic-clonic

1 Definitions, other seizure types and descriptors are listed in the accompanying paper & glossary of terms

2 Due to inadequate information or inability to place in other categories

b ILAE 2017 Classification of Seizure Types Expanded Version 1

Focal Onset Generalized Onset Unknown Onset

Aware
Impaired Motor Motor
Awareness tonic-clonic tonic-clonic
clonic epileptic spasms
Motor Onset tonic Non-Motor
automatisms myoclonic
myoclonic-tonic-clonic behavior arrest
atonic 2
clonic myoclonic-atonic
epileptic spasms 2 atonic
hyperkinetic epileptic spasms
Non-Motor (absence) Unclassified 3
myoclonic
tonic typical
Non-Motor Onset atypical
myoclonic
autonomic
eyelid myoclonia
behavior arrest
cognitive
emotional 1 Definitions, other seizure types and descriptors are listed in the
sensory accompanying paper and glossary of terms
2 Degree of awareness usually is not specified
focal to bilateral tonic-clonic 3 Due to inadequate information or inability to place in other categories

Fig. 1 a Basic seizure classification. b Expanded seizure classification. Figures reproduced with permission from the first author (Robert Fisher) and the
publisher (Wiley) of reference [3]

should only be classified as focal behavior arrest seizure if the arithmetic or other cognitive functions. A focal emotion-
behavior arrest is the dominant feature through the entire sei- al seizure begins with spontaneous fear, anxiety or joy.
zure. A focal cognitive seizure refers to impaired cogni- An emotional seizure may involve involuntary laughing
tion during a seizure. The impairment might affect lan- or crying, each of which might or might not be accom-
guage, spatial perception, and ability to calculate panied by a subjective emotion. Focal sensory seizures
48 Page 4 of 6 Curr Neurol Neurosci Rep (2017) 17:48

can consist of tingling or numbness, visual symptoms, head-nodding, manual, oral-facial, pedaling, pelvic thrusting,
sounds, smells, tastes, vertigo, and hot-cold feelings. An perseveration, running (cursive), sexual, undressing, vocaliza-
unambiguous name, such as a focal sensory seizure, can tion/speech, walking; motor: dysarthria, dystonic, fencers
omit the extra words from a full seizure name: focal posture (figure-of-4), incoordination, Jacksonian, paralysis,
onset non-motor sensory seizure. paresis, versive; sensory: auditory, gustatory, hot-cold sensa-
Focal motor and non-motor seizures are classified by the tions, olfactory, somatosensory, vestibular, visual; laterality:
first prominent sign or symptom, even if the first manifesta- left, right, bilateral. Free text descriptions of seizures are also
tion is not the most prominent feature during the course of a very useful.
seizure. The initial manifestations mark the region or network Seizures evolve as they propagate and recruit brain net-
of origin of the seizure. Impaired awareness is an exception, works not involved at the start of a seizure. A classification
such that a focal seizure is classified as an impaired awareness cannot easily distinguish a unitary seizure type with propaga-
seizure if awareness is impaired at any time during the seizure. tion patterns from sequential but different seizures. Rather,
A second exception is behavior arrest. Brief behavior arrest at this is an issue of clinical diagnosis and one that may require
the start of a seizure often is difficult to discern, so a focal observation of multiple seizures. A stereotyped pattern of
behavior arrest seizure is one for which behavior arrest is the signs and symptoms speak to a single seizure type, perhaps
predominant manifestation throughout the entire seizure. with multiple descriptors and free text appended to the seizure
A seizure classification is far from a complete seizure de- type. Varying patterns of signs and symptoms, accompanied
scription. Words following the declaration of seizure type can by different time intervals between symptoms, might argue for
give crucial information about the seizure. These words may separate multifocal seizures. This issue arises with both old
be drawn from other seizure classification terms, for example, and new seizure classifications.
a focal automatism seizure with right arm tonic activity.
Seizure descriptions also can be drawn from an ILAE- Generalized Onset Seizures
suggested common list of words called descriptors [4].
These descriptors are cognitive: acalculia, aphasia, attention Generalized seizure types are similar to those of the 1981 classi-
impairment, dj vu or jamais vu, dissociation, dysphasia, fication, with a few additions. Generalized onset seizures are not
hallucinations, illusions, memory impairment, neglect, forced characterized by level of awareness, because awareness is usually
thinking, responsiveness impairment; emotional or affective: impaired, although awareness can be hard to assess with myo-
agitation, anger, anxiety, crying (dacrystic), fear, laughing clonic or atonic seizures. The primary division is into generalized
(gelastic), paranoia, pleasure; autonomic: asystole, bradycar- onset motor and non-motor (absence) seizures.
dia, flushing, gastrointestinal, hyper/hypoventilation, nausea A generalized tonic-clonic seizure produces immediate loss
or vomiting, pallor, palpitations, piloerection, respiratory of awareness, accompanied by stiffening of all limbs (tonic
changes, tachycardia; automatisms: aggression, eye-blinking, phase), followed by sustained rhythmic jerking of limbs and

Table 1 Method for classifying a seizure

1. Onset Decide whether seizure onset is focal or generalized, using an 80% confidence level.
2. Awareness For focal seizures, decide whether to classify by degree of awareness or to omit awareness as a classifier.
3. Impaired awareness at A focal seizure is a focal impaired awareness seizure if awareness is impaired at any point during the seizure.
any point
4. Onset predominates Classify a focal seizure by its first prominent sign or symptom. Do not count transient behavior arrest.
5. Behavior arrest A focal behavior arrest seizure shows arrest of behavior as the prominent feature of the entire seizure.
6. Motor/non-motor A focal aware or impaired awareness seizure maybe further subclassified by motor or non-motor characteristics.
Alternatively, a focal seizure can directly be characterized by motor or non-motor characteristics, without specifying
level of awareness. Example, a focal tonic seizure.
7. Optional terms Terms such as motor or non-motor may be omitted when the seizure type is otherwise unambiguous.
8. Additional descriptors It is encouraged to add descriptions of other signs and symptoms, suggested descriptors or free text. These do not alter the
seizure type. Example, focal emotional seizure with tonic right arm activity and hyperventilation.
9. Bilateral vs. generalized Use the term bilateral for tonic-clonic seizures that propagate to both hemispheres and generalized for seizures that
apparently originate simultaneously in both.
10. Atypical absence Absence is atypical if it has slow onset or offset, marked changes in tone or EEG spike-waves at less than 3 per second.
11. Clonic vs. myoclonic Clonic refers to sustained rhythmical jerking and myoclonic to a regular unsustained jerking.
12. Eyelid myoclonia Absence with eyelid myoclonia refers to forced upward jerking of the eyelids during an absence seizure.
Curr Neurol Neurosci Rep (2017) 17:48 Page 5 of 6 48

face (clonic phase). Duration is typically 13 min. The seizure home, neuroimaging, serum antibodies, genetic testing, and
may produce a cry at the start, falling, tongue-biting, and any other useful information. A generalized 3/s spike-wave
incontinence. A generalized clonic seizure is associated with pattern in an EEG will definitively classify a seizure with
bilateral and sustained rhythmical jerking of limbs and/or head activity arrest and staring as a generalized absence seizure,
with no tonic stiffening phase. These seizures most often oc- rather than a focal behavior arrest seizure.
cur in young children. A generalized tonic seizure shows stiff-
ening of all limbs, resolving without a clonic jerking phase. How to Use the Classification
Generalized myoclonic seizures show unsustained irregular
bilateral jerking of limbs, face, eyes, or eyelids. The jerking Table 1, adapted from reference [4], summarizes the practical
of generalized myoclonus may not always be left-right syn- approach to using the 2017 classification system.
chronous, but it occurs on both sides. Some clinicians also
describe negative myoclonus as part of certain myoclonic
seizures, although this may be difficult to distinguish from an Conclusions
atonic seizure or non-epileptic asterixis. A generalized
myoclonic-tonic-clonic is like a tonic-clonic seizure, but it is The 2017 ILAE classification is an evolutionary advance over
preceded by a few myoclonic jerks on both sides of the body. prior classifications, because a classification based on funda-
Such seizures are commonly encountered in people with the mental science is not yet possible. Some seizure types that
syndrome of juvenile myoclonic epilepsy. Generalized were previously only generalized, such as tonic, atonic, myo-
myoclonic-atonic seizures present with a few myoclonic jerks, clonic, clonic, and epileptic spasms, now have focal counter-
followed by a limp drop. Such seizures may be seen in parts. New focal seizure types include automatism, behavior
children with Doose syndrome. A generalized atonic seizure arrest, autonomic, cognitive, and emotional seizures. New
is an epileptic drop attack, with sudden loss of muscle tone generalized seizure types include myoclonic-tonic-clonic,
and strength and a fall to the ground or a slump in a chair. myoclonic absence, and absence with eyelid myoclonia.
Atonic seizures usually last only seconds. Generalized Tonic-clonic, behavior arrest and epileptic spasm seizures
epileptic spasms are brief seizures with flexion at the trunk can be provisionally classified even if onset is unknown.
and flexion or extension of the limbs. Generalized typical Level of awareness is employed as a surrogate for level of
absence is associated with sudden onset of activity cessation, consciousness and optionally (if level of awareness applies
sometimes with eye fluttering and head nodding, or other and is known) subdivides focal seizures into aware and im-
automatic behaviors. If persistent for more than several paired awareness seizures.
seconds, awareness and memory are impaired. Recovery is The seizure classification is not a classification of the epi-
immediate. The EEG during these seizures always shows lepsies or epilepsy syndromes. An epilepsy classification
generalized spike waves. Generalized atypical absence takes into account, not only seizure types, but also etiology,
seizures are similar to typical absence seizures, but may have comorbidities and the broad clinical picture [5]. The 2017
slower onset and recovery and more pronounced changes in seizure classification will take some time to become common
tone. Atypical absence seizures can be difficult to distinguish language, but ultimately should clarify what is meant by a
from focal impaired awareness seizures, but absence seizures particular type of seizure.
usually recover more quickly and the EEG patterns are
different. A seizure with a few jerks and then an absence
seizure is classified as a generalized myoclonic absence Acknowledgements The International League Against Epilepsy support-
seizure. Eyelid myoclonia represents jerks of the eyelids and ed travel and incidental expenses involved in task force meetings to develop
the classification. Task force members were not paid. The current author is
upward deviation of the eyes, often precipitated by closing the
grateful to the Classification Task Force members for their primary input into
eyes or by light. Eyelid myoclonia may be associated with the material of the Epilepsia papers from which this review is adapted. These
absence seizures. Although classifying eyelid myoclonia with members are Helen Cross, Carol DSouza, Jacqueline A. French, Sheryl
non-motor (absence) seizures seems to be an oxymoron, the Haut, Norimichi Higurashi, Edouard Hirsch, Floor E. Jansen, Lieven
Lagae, Solomon L. Mosh, Jukka Peltola, Eliane Roulet Perez, Ingrid E.
most important association is with absence seizures in people
Scheffer, Andreas Schulze-Bonhage, Ernest Somerville, Michael Sperling,
with Jeavon syndrome. Elza Marcia Yacubian, and Sameer M. Zuberi.

Supportive Information
Compliance with Ethical Standards
Determination of seizure type usually can be made from signs
Conflict of Interest Robert S. Fisher is supported by the James and
and symptoms, but supplementary information can help to
Carrie Anderson Research Fund, the Maslah Saul MD Chair, the Susan
classify when such information is available. Supplementary Horngen Fund, and the Steve Chen Fund. He holds stock options in
information may include EEG, smartphone videos from Avails Medical, Smart Monitor, Zeto, and Cerebral Therapeutics.
48 Page 6 of 6 Curr Neurol Neurosci Rep (2017) 17:48

Human and Animal Rights and Informed Consent This article does classification of seizure types, developed in 1981 based upon re-
not contain any studies with human or animal subjects performed by any view of hundreds of seizure videos.
of the authors. 2. Gastaut H. Classification of the epilepsies. Proposal for an interna-
tional classification. Epilepsia. 1969;10(Suppl):1421.
3. Fisher RS, Cross JH, French JA, et al. Operational classification of
seizure types by the International League Against Epilepsy: Position
References Paper of the ILAE Commission for Classification and Terminology.
Epilepsia 2017;58:522530. The new classification of seizure
Papers of particular interest, published recently, have been types.
4. Fisher RS, Cross JH, DSouza C, et al. Instruction manual for the
highlighted as:
ILAE 2017 operational classification of seizure types. Epilepsia
Of importance 2017;531542. A "users manual for the new seizure
Of major importance classification.
5. Scheffer IE, Berkovic S, Capovilla G, et al. ILAE classification of the
1. Proposal for revised clinical and electroencephalographic classifica- epilepsies: Position paper of the ILAE Commission for Classification
tion of epileptic seizures. From the Commission on Classification and Terminology. Epilepsia 2017;58:512521. The new classifica-
and Terminology of the International League Against Epilepsy. tion of the epilepsies.
Epilepsia 1981;22:489501. The basis for the current